Splenic lipids in Gaucher's disease.
Column chromatography (on cellulose, silicic acid, and Florisil) and thin-layer chromatography were employed for the separation and purification of lipid fractions from normal and Gaucher spleens. A new hydrolysis procedure, followed by paper chromatography, was used for identification of sugar moieties. A nonhydrolytic combined colorimetric procedure, with anthrone and orcinol, was used for the estimation of glucose and galactose separately in glycolipids. The limitations of this method were examined. Spleens from two control subjects and three patients with Gaucher's disease have been examined in detail. In all Gaucher spleens, the predominant feature was the massive acccumulation of glucocerebroside; neutral ceramide oligohexoside levels were probably within the normal range, as were other neutral lipids and phospholipids. In one case examined for gangliosides, these were increased twentyfold. One Gaucher spleen, in which others had reported that the stored "cerebroside" contained predominantly lactose as the saccharide moiety, has been examined in detail and it has been established that the stored material was, in fact, glucocerebroside, ceramide lactoside levels not being significantly elevated. In a further nine cases glucose was the major sugar detected in the splenic lipids.
D000293:Adolescent; D000328:Adult; D000596:Amino Acids / Q000378:metabolism; D050260:Carbohydrate Metabolism; D002554:Cerebrosides / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002845:Chromatography; D002855:Chromatography, Thin Layer; D003124:Colorimetry; D005615:Freezing; D005690:Galactose / Q000032:analysis / Q000378:metabolism; D005776:Gaucher Disease / Q000378:metabolism*; D005947:Glucose / Q000032:analysis / Q000378:metabolism; D006017:Glycolipids / Q000378:metabolism; D006027:Glycosides / Q000378:metabolism; D006801:Humans; D007223:Infant; D050356:Lipid Metabolism; D008064:Lipidoses / Q000378:metabolism; D008722:Methods; D008875:Middle Aged; D011309:Preservation, Biological; D013154:Spleen / Q000378:metabolism*
D000596:Amino Acids; D002554:Cerebrosides; D006017:Glycolipids; D006027:Glycosides; D005947:Glucose; D005690:Galactose